Let's talk a little bit about
Huntington's Disease-like syndromes.
These are family of inherited
that closely resemble
So they're called
And they resemble
and that they typically produce
a combination of chorea,
cognitive decline and dementia with
behavioral or psychiatric problems.
Therefore, HDL syndromes:
HDL 1, 2, 3 and 4.
When we think about them,
we can divide them into
HDL 1, 2 and 4 which
are autosomal dominant.
They begin in early to mid adulthood
and have clinical manifestations
that are similar to
HDL 3 is autosomal recessive,
often begins in childhood and patients
have declined in thinking ability,
difficulties with movement,
and seizures in this condition.
Let's talk briefly about
or just DRPLA.
This is a progressive brain disorder
that causes involuntary movements,
mental and emotional problems
and a decline in thinking
so symptoms very similar to what
we see in Huntington's disease.
It is a genetic condition, it's inherited
and an autosomal dominant fashion.
The signs and symptoms we see often differ
between affected children and adults
And patients who have
onset before the age of 20,
we see myoclonus, seizures,
behavioral changes and ataxia,
and that presence of seizures
should prompt a workup for DRPLA.
In patients with onset of
the condition after 20,
we see prominent ataxia,
and deterioration of
that looks very similar
to Huntington's disease.
What about drug induced-chorea?
This may be an acute phenomenon or
a consequence of long term therapy
and there are a number of
drugs that we think about.
All of those drugs increase
dopamine levels within the brain.
Dopamine agonist can result
in drug-induced chorea,
levodopa can result in
We use these for
And the late in the setting
of Parkinson's disease,
we can see patients developing dyskinesias
or extra movements that resemble chorea
and are a complication
of peak dose effects
and that kind of on-off phenomenon
that we see with Parkinson's disease.
We can also see drug-induced chorea
with anticonvulsant medications
and CNS stimulants.
Importantly, tardive dyskinesia
is a subtype of chorea.
It's a specific chorea that occurs
after a selected number of drugs.
When onset of chorea is after
exposure to dopamine agonists,
we call it tardive dyskinesia.
And often patients
with tardive dyskinesia
have prominent oral-buccal-lingual
dyskinesias as an extra movements.
These patients develop
involuntary chorea movements
that typically affect the mouth
tongue, causing random
and stereotyped tongue
protrusion and facial grimacing
that becomes quite debilitating.
How do we treat chorea?
Well, this depends on the type and
the cause and the associated disease.
If the Korea is mild and doesn't interfere
with the patient's daily activities,
we may not need to
In more severe cases we
consider pharmacologic therapy.
reserpine can be used
as well as antipsychotics
like haloperidol and others.
And then in some cases,
we will consider deep brain stimulation.
And then let's talk about
These are dyskinesias,
there's extra movements,
it's a movement disorder
that are paroxysmal.
They come and go.
And that fluctuation is very
different from what we see
in many of the
These are episodic movement disorders
in which there's abnormal movements
that are present only
during the acute attacks.
And there are three forms.
The first is paroxysmal
These are brief
such as chorea, ballismus,
that's triggered by sudden
movements so it's kinesigenic.
The sudden movement initiates
this dyskinetic phenomenon.
These are attacks, they occur in episodes
of dystonic or choreoathetonic movements
precipitated by stress, fatigue,
coffee, alcohol, or menstruation.
So it's not the
movement that initiates
or is the trigger of this
dyskinesia, this extra movement.
It stress, fatigue, coffee,
alcohol or other stressors.
And then exercise
which as the name implies are
dyskinetic extra movements of the body
that occur as a result of
prolonged or vigorous exercise.